1
304A ABSTRACfS JACC February 1995 in the biventricular repair group (not statistically significant by Fischer's exact test, p = 0.27). Only 2 patients have required reoperations, both for residual VSDs. 9 of the Fontan procedures were undertaken in patients who had com- plex anatomy (e.g. AV discordance, unbalanced ventricles, multiple VSDs, atrial isomerism or pulmonary atresia), whereas 14 children of the biventric- ular repair group had non-complex anatomy (p < 0.005). Surgery for double outlet RV with non-committed VSO needs to be tailored to individual patients, but biventricular repair was achieved in a substantial majority of patients with non-complex anatomy with relatively low risk and a low incidence of reoperations. and resealed by incubation at 3JOC for 40 min. For 1I0prost loading. 1I0prost at a concentration 20 Ilg/ml was added to the medium. Quantification of platelet deposition at the site of arterial injury was obtained using indium- 111 labeled platelets infused 1/2 hour prior to balloon injury. Involved arterial segments were excised 2 hours after arterial injury. The results are expressed as platelets per centimeter of artery (graph). Further, preliminary results in an atherosclerotic rabbit model demonstrated a 64% reduction in platelet deposition using i1oprost loaded platelets. Conclusion: Taking advantage of a natural physiologic process after ar- terial injury, this delivery system offers a unique "piggy-back" mechanism of drug delivery. Platelet encapsulation with 1I0prost and other agents deserves further study. 1985-621 Outcome of Anomalous OrigIn of the Left Coronary Artery 1 9 85- 55 1 1985-541 Marshall L. Jacobs, Jack Rychik. John D. Murphy, Susan C. Nicolson, James M. Steven, William I. Norwood. The Children's Hospital of Philadelphia. Philadelphia. PA Norwood's operation provides satisfactory palliation for neonates with hy- poplastic left heart syndrome. The dominant physiologic features of hy- poplastic left heart syndrome, ductal dependency of the systemic circulation and parallel pulmonary and systemic circulations, are shared by a multitude of other less common congenital heart malformations. Theoretically, these should be equally amenable to palliation by Norwood's operation. Since Jan- uary 1990,60 neonates with malformations other than hypoplastic left heart syndrome have undergone initial surgical palliation by Norwood's procedure. Diagnoses include single left ventricle with I-transposition of the great arter- ies (12), critical aortic stenosis (8), complex double outlet right ventricle (81, interrupted aortic arch with ventricular septal defect and subaortic stenosis (7), ventricular septal defect, subaortic stenosis, and coarctation of the aorta (7). aortic atresia with large ventricular septal defect (6), tricuspid atresia with transposition of the great arteries (6). heterotaxy syndrome with subaortic obstruction (3), and other (3). There were 10 hospital deaths and 50 survivors (83% survival). After the introduction of inspired carbon dioxide into the post- operative management protocol (1991),42 of 47 patients survived (89% sur- vival). Mortality is independent of diagnosis, and essentially the same as for hypoplastic left heart syndrome. With minor technical modifications, Nor- wood's operation provides satisfactory initial palliation for a wide variety of malformations characterized by ductal dependency of the systemic circula- tion. in anticipation of either a Fontan procedure or a biventricular repair. Results of Norwood Operation for Lesions other than Hypoplastic Left Heart Syndrome Marcy L. Schwartz, John E. Mayer. Richard A. Jonas. Redmond P. Burke. Steven D. Colan. Children's Hospital, Boston, MA The outcome after surgical repair of anomalous origin of the left coronary artery from the pulmonary trunk (ALCAPA) remains incompletely defined, and transplantation has been advocated for patients with severe left ven- tricular dysfunction. We reviewed our institutional experience with ALCAPA since 1977 in 38 patients whose repair established a dual coronary circulation (7 early cases with LCA ligation were excluded) to assess whether cardiac function predicts outcome, the use of left ventricular assist device (LVAOI, and postoperative complications of the current operative techniques. Surgi- cal techniques included direct coronary reimplantation to the aorta in 11 pts, intrapulmonary baffling (Takeuchi) when the LCA could not reach the aorta di- rectly in 25 pts, and modified Takeuchi in which an extrapulmonary baffle was fashioned from anterior and posterior walls of the pulmonary artery in 2 pts. The operative survival was 91 %,88%, and 100%, respectively (89% overall). All pts who died were <lyr old (3/19 Takeuchi, 1/10 reimplantation). Since its introduction at our institution in 1990, LVAO has been used in 4/16 pts, 1 Takeuchi pt who died and 3 reimplantation pts who all survived. Survival in pts <1 yr old was not different in those with severe dysfunction (short- ening fraction (SF) <15%,18120) compared with moderate dysfunction (SF 16-25%, 4/5). Function returned to normal in all pts for whom followup is known past one year, regardless of the severity of preoperative dysfunction. Postoperative complications for Takeuchi pts included baffle leaks in 6/21 pts who underwent postoperative echocardiogram, supravalvar pulmonary stenosis in 13/20 pts for whom followup is known «25 mmHg in 9 pts, 26-50 mmHg in 2 pts, >50 mmHg in 2 pts requiring reoperation), and progressive aortic regurgitation requiring aortic valve replacement in 1 pI. There were no complications in the two modified Takeuchi pts (6 and 12 mos followup) or in reimplantation pts. Conclusions: (1) The degree of LV dysfunction at presentation does not predict survival in pts < 1yr old. (2) current operative survival for ALCAPA does not justify consideration of transplantation, (3) LVAD may improve survival for some patients and should be available for postoperative management, and (4) supravalvar pulmonary stenosis occurs in a majority of Takeuchi pts, some of whom require reoperation; a modified technique may improve this postoperative complication rate. 1985-631 Do Central Pulmonary Arteries Grow Following Bidirectional Superior Cavopulmonary Anastomosis? Zdenek Slavik, Pavel Horvath, Anthony P. Salmon, Piers E.F. Daubeney, Petr Tax, James L. Monro, Barry R. Keeton, Steven A. Webber, Robert K. Lamb. Wessex CardiC'thoracic Centre, Southampton General Hospital. Southampton. UK: Kardiocentrum, Prague, Czech Republic Surgical Repair of Double Outlet Right Ventricle with Non-committed Ventricular Septal Defect Sabine Kleinert, Tetsuya Sano, Robert G. Weintraub. James L. Wilkinson. Tom R. Karl. Royal Children's Hospital. Melbourne, Australia Wednesday, March 22,1995,9:00 a.m.-11:00 a.m. Ernest N. Morial Convention Center, Hall E Presentation Hour: 10:00 a.m.-11 :00 a.m. The role of bidirectional superior cavopulmonary anastomosis (BCPA) in pal- liation of complex congenital heart defects is now established but little is known about the growth of pulmonary arteries following this operation. Between 1987 and 1993, 74 children underwent BCPA in 2 centres. Of these 21 were further studied by postoperative angiography and are the sub- ject of this investigation. Their median age at BCPA was 23 months (range 1- 108 months). BCPA was undertaken through athoracotomy in 5 and through a midline sternotomy on cardiopulmonary bypass in 16. Competitive sources of pulmonary blood flow were left open in 9 patients. Follow-up angiograms were performed 1-65 months (median 11 months) postoperatively. Z-scores, which standardize pulmonary arteries to body surface area, were calculated from measurement of the diameter immediately prior to the first branching point of each pulmonary artery. The pre- and postoperative bodysurface areas increased from 0.46 m 2 (SD 0.21) to 0.66 m 2 (SO 0.22). The pre- and postoperative Z-scores of the right pulmonary artery were -1.77 (SO 2.63) and -0.38 (SO 1.69) respectively. The left pulmonary artery Z-scores were -0.08 (SO 2.14) and -0.17 (SO 1.31) pre- and post- operatively. In the subset of 13 patients with reduced pulmonary blood flow (0 2 saturation <0.75) preoperatively, the right pulmonary artery grew in excess of somatic growth (mean Z-score -2.63 pre and -0.62 post- operatively; p = 0.003). There was no difference in growth of the pulmonary arteries in patients with and without competitive pulmonary blood flow. Our preliminary results demonstrate that adequate growth of the pul- monary arteries occurs following BCPA in short term follow-up. Pediatric Cardiac Surgery/Postoperative Between January 1978 and June 1993, 32 patients presented for manage- ment of double outlet RVwith non-committed VSD. 29 patients had definitive palliation or repair with 3 early and 1 late deaths (hospital mortality: 10.4%). The VSO type was muscular in 4, inlet in 12 and perimembranous but dis- tant from both great arteries in 16 patients. Complicating anomalies with a major influence on management were present in 23 children (72%). These included aortic arch interruption (3), coarctation of the aorta (4), pulmonary stenosis (11), pulmonary atresia (2). multiple VSOs (3), AV discordance (2), left atrial isomerism (3), unbalanced ventricles (8) and criss-cross AV connection (2) with 11 children having more than one of these lesions. Initial palliation was performed in 20 patients and consisted of pulmonary artery banding in 8 (with repair of an interrupted aortic arch in 2), systemic- pulmonary artery shunt in 9 and repair of coarctation of the aorta in 3. Of these, 17 subsequently had definitive surgery: Fontan procedure in 9 and biventricular repair in 8 (arterial switch in one, intraventricular baffle in 71. 3 patients died following attempted biventricular repair. 12 patients underwent repair as the initial procedure: Fontan procedure in 2 and biventricular repair in 10 (arterial switch in 3 and intraventricular baffle in 71 with no deaths. Thus, of the 29 patients who had definitive surgery, 11 had Fontan procedures and 18 biventricular repair with no death in the Fontan group and 3 early deaths

985-55 Surgical Repair of Double Outlet Right Ventricle with Non-committed Ventricular Septal Defect

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304A ABSTRACfS JACC February 1995

in the biventricular repair group (not statistically significant by Fischer's exacttest, p = 0.27). Only 2 patients have required reoperations, both for residualVSDs. 9 of the Fontan procedures were undertaken in patients who had com­plex anatomy (e.g. AV discordance, unbalanced ventricles, multiple VSDs,atrial isomerism or pulmonary atresia), whereas 14 children of the biventric­ular repair group had non-complex anatomy (p < 0.005).

Surgery for double outlet RV with non-committed VSO needs to be tailoredto individual patients, but biventricular repair was achieved in a substantialmajority of patients with non-complex anatomy with relatively low risk and alow incidence of reoperations.

and resealed by incubation at 3JOC for 40 min. For 1I0prost loading. 1I0prostat a concentration 20 Ilg/ml was added to the medium. Quantification ofplatelet deposition at the site of arterial injury was obtained using indium­111 labeled platelets infused 1/2 hour prior to balloon injury. Involved arterialsegments were excised 2 hours after arterial injury. The results are expressedas platelets per centimeter of artery (graph). Further, preliminary results inan atherosclerotic rabbit model demonstrated a 64% reduction in plateletdeposition using i1oprost loaded platelets.

Conclusion: Taking advantage of a natural physiologic process after ar­terial injury, this delivery system offers a unique "piggy-back" mechanism ofdrug delivery. Platelet encapsulation with 1I0prost and other agents deservesfurther study.

1985-621 Outcome of Anomalous OrigIn of the LeftCoronary Artery

1 985-55 1

1985-541

Marshall L. Jacobs, Jack Rychik. John D. Murphy, Susan C. Nicolson, JamesM. Steven, William I. Norwood. The Children's Hospital of Philadelphia. Philadelphia.PA

Norwood's operation provides satisfactory palliation for neonates with hy­poplastic left heart syndrome. The dominant physiologic features of hy­poplastic left heart syndrome, ductal dependency of the systemic circulationand parallel pulmonary and systemic circulations, are shared by a multitudeof other less common congenital heart malformations. Theoretically, theseshould be equally amenable to palliation by Norwood's operation. Since Jan­uary 1990,60 neonates with malformations other than hypoplastic left heartsyndrome have undergone initial surgical palliation by Norwood's procedure.Diagnoses include single left ventricle with I-transposition of the great arter­ies (12), critical aortic stenosis (8), complex double outlet right ventricle (81,interrupted aortic arch with ventricular septal defect and subaortic stenosis(7), ventricular septal defect, subaortic stenosis, and coarctation of the aorta(7). aortic atresia with large ventricular septal defect (6), tricuspid atresia withtransposition of the great arteries (6). heterotaxy syndrome with subaorticobstruction (3), and other (3). There were 10 hospital deaths and 50 survivors(83% survival). After the introduction of inspired carbon dioxide into the post­operative management protocol (1991),42 of 47 patients survived (89% sur­vival). Mortality is independent of diagnosis, and essentially the same as forhypoplastic left heart syndrome. With minor technical modifications, Nor­wood's operation provides satisfactory initial palliation for a wide variety ofmalformations characterized by ductal dependency of the systemic circula­tion. in anticipation of either a Fontan procedure or a biventricular repair.

Results of Norwood Operation for Lesions otherthan Hypoplastic Left Heart Syndrome

Marcy L. Schwartz, John E. Mayer. Richard A. Jonas. Redmond P. Burke. StevenD. Colan. Children's Hospital, Boston, MA

The outcome after surgical repair of anomalous origin of the left coronaryartery from the pulmonary trunk (ALCAPA) remains incompletely defined,and transplantation has been advocated for patients with severe left ven­tricular dysfunction. We reviewed our institutional experience with ALCAPAsince 1977 in 38 patients whose repair established a dual coronary circulation(7 early cases with LCA ligation were excluded) to assess whether cardiacfunction predicts outcome, the use of left ventricular assist device (LVAOI,and postoperative complications of the current operative techniques. Surgi­cal techniques included direct coronary reimplantation to the aorta in 11 pts,intrapulmonary baffling (Takeuchi) when the LCA could not reach the aorta di­rectly in 25 pts, and modified Takeuchi in which an extrapulmonary baffle wasfashioned from anterior and posterior walls of the pulmonary artery in 2 pts.The operative survival was 91 %,88%, and 100%, respectively (89% overall).All pts who died were <lyr old (3/19 Takeuchi, 1/10 reimplantation). Sinceits introduction at our institution in 1990, LVAO has been used in 4/16 pts,1 Takeuchi pt who died and 3 reimplantation pts who all survived. Survivalin pts <1 yr old was not different in those with severe dysfunction (short­ening fraction (SF) <15%,18120) compared with moderate dysfunction (SF16-25%, 4/5). Function returned to normal in all pts for whom followup isknown past one year, regardless of the severity of preoperative dysfunction.Postoperative complications for Takeuchi pts included baffle leaks in 6/21pts who underwent postoperative echocardiogram, supravalvar pulmonarystenosis in 13/20 pts for whom followup is known «25 mmHg in 9 pts, 26-50mmHg in 2 pts, >50 mmHg in 2 pts requiring reoperation), and progressiveaortic regurgitation requiring aortic valve replacement in 1 pI. There were nocomplications in the two modified Takeuchi pts (6 and 12 mos followup) orin reimplantation pts.

Conclusions: (1) The degree of LV dysfunction at presentation does notpredict survival in pts < 1yr old. (2) current operative survival for ALCAPA doesnot justify consideration of transplantation, (3) LVAD may improve survivalfor some patients and should be available for postoperative management,and (4) supravalvar pulmonary stenosis occurs in a majority of Takeuchi pts,some of whom require reoperation; a modified technique may improve thispostoperative complication rate.

1985-631

Do Central Pulmonary Arteries Grow FollowingBidirectional Superior CavopulmonaryAnastomosis?

Zdenek Slavik, Pavel Horvath, Anthony P. Salmon, Piers E.F. Daubeney, Petr Tax,James L. Monro, Barry R. Keeton, Steven A. Webber, Robert K. Lamb. WessexCardiC'thoracic Centre, Southampton General Hospital. Southampton. UK:Kardiocentrum, Prague, Czech Republic

Surgical Repair of Double Outlet Right Ventriclewith Non-committed Ventricular Septal Defect

Sabine Kleinert, Tetsuya Sano, Robert G. Weintraub. James L. Wilkinson. TomR. Karl. Royal Children's Hospital. Melbourne, Australia

Wednesday, March 22,1995,9:00 a.m.-11:00 a.m.Ernest N. Morial Convention Center, Hall EPresentation Hour: 10:00 a.m.-11 :00 a.m.

The role of bidirectional superior cavopulmonary anastomosis (BCPA) in pal­liation of complex congenital heart defects is now established but little isknown about the growth of pulmonary arteries following this operation.

Between 1987 and 1993, 74 children underwent BCPA in 2 centres. Ofthese 21 were further studied by postoperative angiography and are the sub­ject of this investigation. Their median age at BCPA was 23 months (range 1­108 months). BCPA was undertaken through a thoracotomy in 5 and througha midline sternotomy on cardiopulmonary bypass in 16. Competitive sourcesof pulmonary blood flow were left open in 9 patients. Follow-up angiogramswere performed 1-65 months (median 11 months) postoperatively. Z-scores,which standardize pulmonary arteries to body surface area, were calculatedfrom measurement of the diameter immediately prior to the first branchingpoint of each pulmonary artery.

The pre- and postoperative bodysurface areas increased from 0.46 m2 (SD0.21) to 0.66 m2 (SO 0.22). The pre- and postoperative Z-scores of the rightpulmonary artery were -1.77 (SO 2.63) and -0.38 (SO 1.69) respectively. Theleft pulmonary artery Z-scores were -0.08 (SO 2.14) and -0.17 (SO 1.31) pre­and post- operatively. In the subset of 13 patients with reduced pulmonaryblood flow (02 saturation <0.75) preoperatively, the right pulmonary arterygrew in excess of somatic growth (mean Z-score -2.63 pre and -0.62 post­operatively; p = 0.003). There was no difference in growth of the pulmonaryarteries in patients with and without competitive pulmonary blood flow.

Our preliminary results demonstrate that adequate growth of the pul­monary arteries occurs following BCPA in short term follow-up.

Pediatric Cardiac Surgery/Postoperative

Between January 1978 and June 1993, 32 patients presented for manage­ment of double outlet RVwith non-committed VSD. 29 patients had definitivepalliation or repair with 3 early and 1 late deaths (hospital mortality: 10.4%).

The VSO type was muscular in 4, inlet in 12 and perimembranous but dis­tant from both great arteries in 16 patients. Complicating anomalies with amajor influence on management were present in 23 children (72%). Theseincluded aortic arch interruption (3), coarctation of the aorta (4), pulmonarystenosis (11), pulmonary atresia (2). multiple VSOs (3), AV discordance (2), leftatrial isomerism (3), unbalanced ventricles (8) and criss-cross AV connection(2) with 11 children having more than one of these lesions.

Initial palliation was performed in 20 patients and consisted of pulmonaryartery banding in 8 (with repair of an interrupted aortic arch in 2), systemic­pulmonary artery shunt in 9 and repair of coarctation of the aorta in 3. Ofthese, 17 subsequently had definitive surgery: Fontan procedure in 9 andbiventricular repair in 8 (arterial switch in one, intraventricular baffle in 71. 3patients died following attempted biventricular repair. 12 patients underwentrepair as the initial procedure: Fontan procedure in 2 and biventricular repairin 10 (arterial switch in 3 and intraventricular baffle in 71 with no deaths. Thus,of the 29 patients who had definitive surgery, 11 had Fontan procedures and18 biventricular repair with no death in the Fontan group and 3 early deaths